Background

Inflammatory bowel disease (IBD) in children has a more aggressive disease course when compared to adults. Restorative proctocolectomy with ileal pouch anal anastomosis (IPAA) has become the treatment of choice in paediatric patients who have failed medical therapy or diagnosed with familial adenomatous polyposis (FAP). A higher incidence of postoperative complications is expected in IBD, since these patients are in a more disease-affected state with more medication use. However, data regarding the long-term outcomes in these patients compared to FAP patients are limited. The aim of our study is to compare the long term outcomes between paediatric patients with IBD and FAP who underwent restorative proctocolectomy with IPAA.

Methods

In a retrospective study, 53 (27 ulcerative colitis, 3 indeterminate colitis, 4 Crohn's disease, 19 FAP) consecutive children under the age of 19 that underwent IPAA surgery between February 1991 and September 2014 were included. Pouch outcomes of paediatric IBD patients with a median age of 16 years (IQR, 14 - 17) were compared to paediatric FAP patients with a median age of 16.5 years (IQR, 15-17). The mean follow-up was 4.1 years (range, 1.2 - 22.3 years) and was comparable between both groups.

Results

IBD patients were in a more disease-affected state than FAP with an ASA score of more than 2 (73.2% vs 14.7%, p=0.001). In line with this, 48.3% of the IBD patients were treated with steroids and 3.3% with biologicals within 3 months before surgery compared to no treatment in FAP. The majority of IBD patients had a pouch procedure in multiple stages, that is an initial subtotal colectomy followed by completion proctectomy with IPAA at a later stage (76.5% vs 36.8%, p=0.007). Interestingly, the short-term anastomotic leak rate was comparable between IBD and FAP (15.8% vs 17.6%, p=0.863). Although not significant, fewer IBD patients had strictures compared to FAP (3.8% vs 16.7%, p=0.146). IBD patients had higher fistula (19.2% vs 0%, p=0.048) and pouchitis rates (57.7% vs 11.1%, p=0.002) compared to FAP patients. However, long-term Results showed comparable pouch failure rates between both groups (15.8% vs 11.8%, p=0.691).

Conclusion

Surgeons perform more multi-staged procedures in paediatric IBD patients in order to wean of medication and restore their nutritional state before restoring the continuity. However, postoperative complications rates are high which is possibly due to the inflammatory state of the resected colon in IBD patients. On the long term, pouch failure rates remain comparable between both groups. Although these complications do not alter the clinical course, attention should be paid on lowering the high fistula and pouchitis rates since it may negatively affect a child's life.